Steroid responsive familial neuropathy with liability to pressure palsies.
Autosomal dominant motor and sensory neuropathy with liability to pressure palsies was studied in three members of the same family. Only one of two monozygotic twin sisters was clinically affected. She developed unilateral peroneal palsy twenty minutes following local pressure. Electromyography revealed a weak intermediate innervation pattern with very rapid action potentials in the right anterior lower leg muscle. A 25-70 per cent reduction of motor and sensory conduction velocity was recorded in the clinically unaffected twin sister and in the father. The electrophysiological findings in the mother were normal. The sural nerve biopsy revealed "sausage-like" formations. The palsy persisted for two months and disappeared after eight weeks of fluocortolon treatment. It is possible that the myelin sheaths acted as antigen.
Barisić
N
N
Department of Pediatrics, University Hospital Rebro, Zagreb, Yugoslavia.
Skarpa
D
D
Jusić
A
A
Jadro-Santel
D
D
D001706:Biopsy; D002648:Child; D004200:Diseases in Twins / Q000235:genetics*; D004576:Electromyography / Q000187:drug effects; D005260:Female; D005448:Fluocortolone / Q000008:administration & dosage*; D015417:Hereditary Sensory and Motor Neuropathy / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D006801:Humans; D009046:Motor Neurons / Q000187:drug effects; D009408:Nerve Compression Syndromes / Q000150:complications* / Q000473:pathology; D009431:Neural Conduction / Q000187:drug effects; D010543:Peroneal Nerve / Q000187:drug effects* / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology; D014430:Twins, Monozygotic / Q000235:genetics
D005448:Fluocortolone